Pretibial epidermolysis bullosa is a rare variant of hereditary epidermolysis bullosa characterized by the delayed onset of lesions and their localization. We present two cases, a 35-year-old woman and a 21-year-old man. They clinically had pruritus, nodular prurigo-like or lichenified lesions, violaceous scarring, milia, nail dystrophy and in one case albopapuloid lesions on the trunk. Physical signs of disease were first noticed between 3 and 9 years of age. Histologic examination showed a subepidermal blister with milia formation, and a mild mixed inflammatory infiltrate. These patients represent an nusual, poorly recognized form of dystrophic epidermolysis bullosa with features that resemble a variety of acquired dermatoses.